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Systemic Sclerosis (Scleroderma)
Multi-system autoimmune disease characterized by vasculopathy and progressive fibrosis of skin and internal organs
3
Systemic Sclerosis ... (Scleroderma) ... 3 Subtypes of Scleroderma ... Sclerosis sine scleroderma ... telangiectasias, poikiloderma
ANA Patterns and Associated Rheumatic Diseases:
 • Homogeneous: Systemic lupus erythematosus (SLE), Mixed connective tissue disease
• Homogeneous: Systemic ... sclerosis or scleroderma ... sclerosis/scleroderma ... sclerosis/scleroderma ... sclerosis/scleroderma
Systemic Sclerosis (Scleroderma)
Limited - CREST:
 • Calcinosis
 • Raynaud phenomenon
 • Esophageal dysmotility
 • Sclerodactyly
 •
Systemic Sclerosis ... (Scleroderma) ... 10% - 15% • Scleroderma ... @AnnKumfer #Systemic ... #Sclerosis #Scleroderma
Antinuclear Antibodies (ANA)
ANA or Anti-NucIear Antibody refers to antibodies against antigens in the nucleus → like
sclerosis or scleroderma ... sclerosis/scleroderma ... sclerosis/scleroderma ... sclerosis/scleroderma ... Sclerosis (scleroderma
Cutaneous and Systemic Findings in Dermatomyositis
Cutaneous Findings:
 - Heliotrope sign and edema
 - Nail-fold changes
 -
Cutaneous and Systemic ... Photodistributed eruption - Poikiloderma ... Holster sign Systemic ... #Cutaneous #Systemic
Scleroderma Mimics
 • Scleredema
 • Scleromyxedema (papular mucinosis)
 • Eosinophilic Fasciitis
 • Nephrogenic Systemic Fibrosis
 •
Scleroderma Mimics ... • Scleredema ... • Nephrogenic Systemic ... @MithuRheum #Scleroderma
ILD of Known Cause - Differential Diagnosis
Exposure:
 • Occupational: Asbestosis, Silicosis, Hypersensitivity, Pneumonitis
 • Treatment Related:
Chemotherapeutic Systemic ... Tissue Disease: RA, Scleroderma
Scleroderma Like Conditions - Differential Diagnosis
Eosinophilic Fasciitis:
 - Orange peel induration (peau d'orange) of proximal extremities
Scleroderma Like ... treatment Nephrogenic Systemic ... reduced incidence Scleredema ... - Can produce scleroderma-like ... Chiefs @jmcchiefs #Scleroderma
Nail Disorders secondary to Systemic Diseases
Nail Fold Abnormality
 • SLE
 • Scleroderma
 • Dermatomyositis
Koilonychia - Spoon-shaped
Disorders secondary to Systemic ... Abnormality • SLE • Scleroderma ... Grooves • Any systemic ... NailDisorders #Systemic
Classification of Vasculitis 
Vasculitis of large vessels 
 - Arteritis temporalis (giant-cell arteritis) 
 - Takayasu
central nervous system ... disease - systemic ... diseases - scleroderma